Donald L. Price, M.D.
Neurobiology of human diseases (particularly neurodegenerative disorders), genetically engineered models, and experimental therapeutics
Investigations involve: clinical‑pathological-biochemical features of human illnesses, particularly like Alzheimer's Disease (AD), amyotrophic lateral sclerosis (ALS), and Parkinson’s Disease (PD); analyses of the processes leading to selective vulnerability of specific neuronal systems in these diseases; creation and characterization of transgenic mice expressing mutant genes implicated in these illnesses; investigations of the mechanisms whereby mutations cause neural abnormalities in vitro and in vivo; analyses of the outcomes of targeting specific genes to assess the functions of gene products in disease and to identify new therapeutic targets; and testing of experimental treatments in animal models to facilitate new therapies for human illnesses.
- Wong PC, Price DL, Cai H. The brain's susceptibility to amyloid plaques. Science. 2001 Aug 24;293(5534):1434
- Cai H, Wang Y, McCarthy D, Wen H, Borchelt DR, Price DL, Wong PC. BACE1 is the major b-secretase for generation of Ab peptides by neurons. Nat Neurosci. 2001 Mar;4(3):233-4.
- Wong PC, Waggoner D, Subramaniam JR, Tessarollo L, Bartnikas TB, Culotta VC, Price DL, Rothstein J, Gitlin JD. Copper chaperone for superoxide dismutase is essential to activate mammalian Cu/Zn superoxide dismutase. Proc Natl Acad Sci U S A. 2000 Mar 14;97(6):2886-91.
- Wong PC, Cai H, Borchelt DR and Price DL: Genetically engineered mouse models of neurodegenerative disease. Nature Neurosci., 5:633-639, 2002.
- Subramaniam JR, Lyons E, Liu J, Bartinakas T, Rothstein J, Price DL, Cleveland DW, Gitlin JD and Wong PC:� Mutant SOD1 causes motor neuron disease independent of CCS-mediated copper loading. Nat. Neurosci., 5:301-307, 2002.
- Lee MK, Stirling W, Xu Y, Xu X, Qui D, Mandir AS, Dawson TM, Copeland NG, Jenkins NA, Price DL:� Human a-Synuclein harboring familial Parkinson's disease linked Ala53Thr mutation causes neurodegenerative disease with a-Synuclein aggregation in transgenic mice.� PNAS, 99:8968-8973, 2002.
- Li T, Ma G, Cai H, Price DL, Wong PC: Nicastrin is required for assembly of presenilin/g-secretase complexes to mediate Notch signaling, and processing and trafficking of b-APP in mammals. J. of Neuroscience, 23: 3272-3277, 2003.
- Wong PC, Waggoner D, Subramaniam J, Tessarollo L, Bartnikas TB, Culotta VC, Price DL, Rothstein J and Gitlin JD: Copper chaperone for superoxide dismutase is essential to activate mammalian Cu/Zn superoxide dismutase.� Proc. Natl. Acad. Sci. U.S.A. 97:� 2886-2891, 2000.